Children with Steroid-sensitive Nephrotic Syndrome Come of Age: Long-term Outcome

• Published in: The Journal of pediatrics 2005-08, Vol.147 (2), p.202-207
• Main Authors: Rüth, Eva-Maria, Kemper, Markus J., Leumann, Ernst P., Laube, Guido F., Neuhaus, Thomas J.
• Format: Article
• Language: English
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; Antineoplastic Agents, Alkylating - adverse effects; Biological and medical sciences; Child, Preschool; Chlorambucil - adverse effects; Cyclophosphamide - adverse effects; Female; Follow-Up Studies; General aspects; Glomerulonephritis; Humans; Infertility, Male - chemically induced; Kidney Function Tests; Male; Medical sciences; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; Nephrotic Syndrome - drug therapy; Nephrotic Syndrome - physiopathology; Obesity - chemically induced; Prognosis; Recurrence
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2005.03.050

Long-term outcome of steroid-sensitive idiopathic nephrotic syndrome (SSNS) in children is usually considered benign, although data on follow-up into adulthood are scarce. The aim of this study was to investigate adults who had childhood SSNS regarding their relapse rate, growth, and renal and extrarenal morbidity. Adult patients (n = 42, 26 males) were evaluated at a median age of 28.0 (18.1 to 46.9) years and a median follow-up of 22.0 (2.9 to 39.0) years since diagnosis. Fourteen of 42 (33%) patients relapsed in adulthood. The number of relapses during childhood and adolescence and a complicated course—administration of steroid-sparing medication such as cyclophosphamide, chlorambucil, and cyclosporin A—were identified as risk factors. Final adult height (median SD score −0.4, range −3.3 to +1.3) and body mass index (BMI) were normal. Renal function was normal in all patients, and overall morbidity was low. Only eight patients (three males) had children. Cytotoxic therapy was identified as a major factor contributing to childlessness. Relapses in adulthood were common in pediatric patients with SSNS. Growth and renal function were normal, and overall morbidity was low. Yet, transition to an adult nephrologist is recommended for all children with relapsing SSNS.